Abstract

BackgroundAn effort to unify the variability of juvenile idiopathic arthritis (JIA) phenotypes was initiated in 1994 resulting in the current categories of systemic JIA, oligoarthritis (persistent or extended), rheumatoid factor (RF)-negative polyarthritis, RF-positive polyarthritis, psoriatic JIA, and enthesitis related arthritis.Recently, EULAR has proposed a definition of difficult-to-treat rheumatoid arthritis (D2TRA) and the concept was later extended to spondyloarthritis and psoriatic arthritis.ObjectivesThe aim of this study was to describe difficult-to-treat JIA (D2TJIA) at adulthood based on the EULAR definition of D2TRA.MethodsWe stratified consecutive JIA patients treated at Cochin Hospital in Paris into two groups, a D2TJIA group and a non-D2TJIA group. Based on EULAR definition of D2TRA, we defined D2TJIA as JIAs failing at least two targeted therapies, with a different mechanism of action.ResultsIn total, we identified 18 D2TJIA patients (mean age 27.4 years, 67% female) and 88 non-D2TJIA patients (mean age 29.1 years, 70% female). Regarding JIA subtypes, we observed significantly more systemic JIA in the D2T group (39% vs 14%, p=0.02). D2TJIA patients were more likely to have erosions on X-rays (82% vs. 55%, p=0.038). Regarding auto-antibodies, positive anti-CCP (6.7% vs. 35%, p=0.048) were less common in D2TJIA than in non-D2TJIA patients, and no difference was observed for RF and anti-nuclear antibody positivity (Table 1).Compared with non-D2TJIA, patients with D2TJIA had a significantly reduced duration of exposure to the first line of bDMARD (6.5 vs 32 months, p<0.01) (Table 1).Table 1.Characteristics of JIA patientsNon-D2TJIA (n = 88)D2TJIA (n = 18)pAge, mean (SD)29.1 (3.53)27.4 (4.50)0.14Female, n (%)62 (70%)12 (67%)0.75JIA type, n (%)Systemic JIARF-positive polyarthritisRF-negative polyarthritisPersistent oligoarthritisExtended oligoarthritisPsoriatic JIAEnthesitis related arthritis12 (14)18 (20)13 (15)19 (22)4 (5)1 (1)21 (24)7 (39)3 (17)3 (17)3 (17)1 (5)1 (5)1 (5)0.0210.70.810.30.1Age at diagnosis, median [Q25-75]9.00 [4.00; 13.0]7.00 [3.00; 8.00]0.11Duration of bDMARD 1 (in months), median [Q25-75]32.0 [16.0; 79.0]6.50 [4.25; 31.5]<0.01Duration of bDMARD 2 (in months), median [Q25-75]21.0 [7.00; 50.2]10.0 [4.00; 23.2]0.17Duration of bDMARD 3 (in months), median [Q25-75]6.00 [2.50; 19.0]13.0 [4.00; 30.0]0.32Duration of bDMARD 4 (in months), median [Q25-75]5.00 [1.00; 28.5]6.00 [4.00; 15.0]0.67Number of used bDMARD, median [Q25-75]2.00 [1.00; 2.00]4.00 [3.00; 4.00]<0.001Prosthesis, n (%)11 (12%)5 (28%)0.14Coxitis, n (%)27 (35%)9 (53%)0.16Inflammatory bowel disease, n (%)1 (1.2%)0 (0%)1Psoriasis, n (%)4 (4.7%)2 (11%)0.28Uveitis, n (%)22 (26%)3 (17%)0.55Erosions, n (%)47 (55%)14 (82%)0.038Anti-nuclear antibody positive, n (%)27 (44%)5 (33%)0.47Anti-CCP positive, n (%)17 (35%)1 (6.7%)0.048RF positive, n (%)21 (35%)4 (25%)0.45ConclusionOur work is the first to describe D2TJIA in France. It seemed interesting to raise the question of whether D2T JIA exists, and if so, what could be its potential definition and management. This may serve as a hypothesis and a basis for future reflections.As observed in rheumatoid arthritis, we report significantly more patients with erosions on X-ray in the D2TJIA group compared to the non-D2TJIA patients which supports such stratification. However, D2TJIA patients did not differ from non-D2TJIA patients in terms of demographic data.Interestingly, patients with D2TJIA had a significantly reduced duration of exposure to the first line of bDMARD suggesting early treatment escape and supporting the importance of temporality in defining difficult-to-treat patients.Our work suggests the relevance of identifying patients with D2TJIA. As showed in other inflammatory rheumatic diseases, this can open the door to a better stratification and personalized management. The definition of D2TJIA needs to be validated and temporality seems to be an essential element to consider.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

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